biometals in rare neurodegenerative disorders of childhood
Clicks: 261
ID: 164756
2013
Article Quality & Performance Metrics
Overall Quality
Not rated
Combines reader engagement with the AI quality analysis. This
article has not been analysed, so there is no overall score —
reader engagement is measured and shown alongside.
Reader Engagement
Popular Article
68.6
/100
261 views
216 readers
Trending
AI Quality Assessment
Not analyzed
Readership in this journal
PopularRanked #140 of 234 articles by views in Frontiers in chemistry
Most read
Least read
Bar heights use a square-root scale. Only the 120 most-read articles are drawn; the journal has 234 in total.
Mint this article as an NFT
Not yet mintedCreate a permanent, verifiable on-chain record of this article on the Scimatic Network. The NFT is held in your Journament account, and you can withdraw it to your own wallet at any time.
5
SUSD
one-off · no wallet required
Abstract
Copper, iron and zinc are just three of the main biometals critical for correct functioning of the central nervous system. They have diverse roles in many functional processes including but not limited to enzyme catalysis, protein stabilisation, and energy production. The range of metal concentrations within the body is tightly regulated and when the balance is perturbed, debilitating effects ensue. Homeostasis of brain biometals is mainly controlled by various metal transporters and metal sequestering proteins. The biological roles of biometals are vastly reviewed in the literature with a large focus on the connection to neurological conditions associated with ageing. Biometals are also implicated in a variety of debilitating inherited childhood disorders, some of which arise soon following birth or as the child progresses into early adulthood. This review acts to highlight what we know about biometals in childhood neurological disorders such as Wilson’s disease, Menkes disease, neuronal ceroid lipofuscinoses and neurodegeneration with brain iron accumulation. Also discussed are some of the animal models available to determine the pathological mechanisms in these childhood disorders, which we hope will aid in our understanding of the role of biometals in disease and in attaining possible therapeutics in the future.
| Reference Key |
parker2013frontiersbiometals
Use this key to autocite in the manuscript while using
SciMatic Manuscript Manager or Thesis Manager
|
|---|---|
| Authors | ;Sarah Jan Parker;Jari eKoistinaho;Anthony Robert White;Katja Marika Kanninen;Katja Marika Kanninen |
| Journal | Frontiers in chemistry |
| Year | 2013 |
| DOI |
10.3389/fnagi.2013.00014
|
| URL | |
| Keywords |
Citations
No citations found. To add a citation, contact the admin at info@scimatic.org
Comments
No comments yet. Be the first to comment on this article.